Blast and Bursts: Unveiling Splenic Rupture in Blastic Plasmacytoid Dendritic Cell Neoplasia.

Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) is a rare hematologic cancer, accounting for less than 1% of acute leukemias in the U.S. Diagnosis involves detecting markers like CD123, CD4, CD56, TCL1, and TCF4. Treatment typically involved acute leukemia therapies, but Tagraxofusp, a targeted therapy, was recently approved. Despite advancements, prognosis remains grim, with a median survival of around 1 year. Atraumatic splenic rupture (ASR) is a rare complication of this condition, with only five cases reported from 1994 to 2018. Here we present a case of BPDCN complicated by ASR. This case emphasizes the challenges of diagnosing and treating BPDCN, noting its rarity and absence of standard therapy. Tagraxofusp has shown promising results but presents safety concerns like capillary leak syndrome, particularly in elderly patients with comorbidities.

Non-Secretory Multiple Myeloma Associated With High-Risk Phenotype and Complex Cytogenetics Including t(8;22).

Multiple myeloma (MM) is a plasma cell dyscrasia which is typically characterized by identifiable paraprotein in the blood or urine. However, the minority of patients in whom paraprotein cannot be identified are designated non-secretory MM (NSM). Evaluation of treatment response is more difficult in these patients as paraprotein levels cannot be followed. A dearth of clinical trials including these patients exists because of an inability to measure response by classical serum and urine measurement mechanisms as well as seemingly decreased overall survival compared to secretory MM. NSM is subdivided into four subgroups: "non-producers", "true non-secretors", "oligosecretors" and "false non-secretors". The "non-producers" phenotype is associated with more aggressive disease course. Translocations such as those involving the proto-oncogene c-MYC (chromosome 8) and the lambda light chain gene IGL (chromosome 22) - more commonly associated with Burkitt lymphoma - are rare in MM. We describe a 60-year-old male with NSM who was identified as having multiple high-risk features including complex cytogenetics and a non-producer phenotype, which are features not considered in conventional MM staging and risk stratification. This case highlights the need for awareness of phenotypes and cytogenetics associated with higher clinical risk that are not included in the revised International Staging System.

Influence of age and gender on autonomic regulation of heart.

Heart rate variability (HRV) is a non-invasive method to measure cardiac autonomic function. Impairments in HRV have been proposed as independent risk factor for increased cardiac mortality and morbidity. Cardio protective phenomenon in females has been hypothesized to be due to differential autonomic tone. Age related loss of vagal control has been reported as predisposing factor for the development of cardiovascular disease. In this study we assessed effect of age and gender on autonomic regulation of heart in healthy volunteers. HRV data of 189 subjects (114 males and 75 females) were analyzed in time and frequency domains using customized program. Artifact free 5 min electrocardiogram segment was used for analysis. It was ensured that none of the subject had medical illness such as diabetes, hypertension, thyroid disorders, cardiac disorders, diseases potentially related with autonomic neuropathy and major psychiatric illness by careful history and clinical examination. HRV recordings were done under standard laboratory condition. On correlation analysis SDNN, RMSSD, total power negatively correlated with age suggesting reduced autonomic regulation of heart with increase in age (SDNN: r = -0.444, p < 0.01; RMSSD: r = -0.552, p < 0.01; total power: r = -0.474, p < 0.01); similarly High frequency power (HF.nu) negatively correlated with age (r = -0.167, p = 0.02), denoting loss of vagal tone with aging. LF/HF ratio correlated positively with age (r = 0.19, p < 0.01) suggesting a relative increase of sympathetic activity with increase in age. On multiple regression analysis to control for effect of age and heart rate while comparing males and females, LF.nu showed significant reduction suggesting lower sympathetic tone in females (ß = -6.64; p < 0.01) and HF.nu showed increase at trend level (ß = 4.47; p = 0.053). In conclusion, there is overall reduction in autonomic control of heart with increase in the age. Sympathetic tone predominates and vagal tone diminishes with aging process. Females showed greater vagal tone than male. This differential autonomic tone indicate age, gender related predisposition to cardiovascular disease.

Symptomatic mature teratoma of the lumbar spine: A case report.

BACKGROUND: Teratomas are a unique family of tumors derived from two or more of the three embryonic layers: endoderm, mesoderm, and ectoderm. Mature teratomas are comprised the most well-differentiated tissue types and may contain skin, hair, teeth, smooth muscle, respiratory tissues, etc. Infrequently, mature teratomas may be found within the central nervous system and, in exceedingly rare cases, may be occur within the spinal cord itself (i.e., intramedullary/intradural). CASE DESCRIPTION: A 78-year-old female presented with a subacute progressive lower extremity paraparesis. The MR revealed a cystic 81 × 30 × 25 mm intradural/intramedullary spinal mass involving the distal conus with exophytic extension into the L1-L4 spinal canal. Following surgical intervention consisting of a L1-L4 laminectomy, the lesion was largely removed. Pathology of the mass confirmed a large mature teratoma containing a multilobulated cyst that intraoperatively compressed the conus and cauda equina. Immediately postoperatively, the patient significantly improved neurologically. However, on postoperative day 2, she acutely developed a change in mental status with the left gaze preference and hemiparesis. CT brain in the acute setting showed no evidence of causative pathology and subsequent MR brain was unremarkable. The patient's neurologic deficits progressively improved leading to eventual discharge. CONCLUSION: Intrathecal intramedullary/extramedullary mature teratomas of the conus that results in subacute cauda equina syndromes are rare. The differential diagnosis for such lesions exophytic to the conus must include mature teratomas which, though rare, may be readily resected resulting in generally favorable outcomes.